Bullous Systemic Lupus Erythematosus With Autoantibodies Recognizing Multiple Skin Basement Membrane Components, Bullous Pemphigoid Antigen 1, Laminin-5, Laminin-6, and Type VII Collagen

Chan, Lawrence S.; Lapiere, Jean Christophe; Chen, Mei; Traczyk, Tom; Mancini, Anthony J.; Paller, Amy S.; Woodley, David T.; Marinkovich, M. Peter

doi:10.1001/archderm.135.5.569

Cited by 113 publications

(87 citation statements)

References 35 publications

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“…14 Mutations or deficiencies of collagen type VII as in bullous systemic lupus erythematosus or dystrophic forms of EB 15 result in a paucity of collagen type VII anchoring collagen fibrils 16 leading to skin fragility and extensive blistering. Patients with skin basement membrane disorders constantly suffer from severe blisters and recurring wounds throughout the body and associated infections.…”

Section: Introductionmentioning

confidence: 99%

Superficial Dermal Fibroblasts Enhance Basement Membrane and Epidermal Barrier Formation in Tissue-Engineered Skin: Implications for Treatment of Skin Basement Membrane Disorders

Varkey

Ding

Tredget

2013

Tissue Engineering Part A

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Basement membrane is a highly specialized structure that binds the dermis and the epidermis of the skin, and is mainly composed of laminins, nidogen, collagen types IV and VII, and the proteoglycans, collagen type XVIII and perlecan, all of which play critical roles in the function and resilience of skin. Both dermal fibroblasts and epidermal keratinocytes contribute to the development of the basement membrane, and in turn the basement membrane and underlying dermis influence the development and function of the epidermal barrier. Disruption of the basement membrane results in skin fragility, extensive painful blistering, and severe recurring wounds as seen in skin basement membrane disorders such as epidermolysis bullosa, a family of life-threatening congenital skin disorders. Currently, there are no successful strategies for treatment of these disorders; we propose the use of tissue-engineered skin as a promising approach for effective wound coverage and to enhance healing. Fibroblasts and keratinocytes isolated from superficial and deep dermis and epidermis, respectively, of tissue from abdominoplasty patients were independently cocultured on collagen-glycosaminoglycan matrices, and the resulting tissue-engineered skin was assessed for functional differences based on the underlying specific dermal fibroblast subpopulation. Tissue-engineered skin with superficial fibroblasts and keratinocytes formed a continuous epidermis with increased epidermal barrier function and expressed higher levels of epidermal proteins, keratin-5, and E-cadherin, compared to that with deep fibroblasts and keratinocytes, which had an intermittent epidermis. Further, tissue-engineered skin with superficial fibroblasts and keratinocytes formed better basement membrane, and produced more laminin-5, nidogen, collagen type VII, compared to that with deep fibroblasts and keratinocytes. Overall, our results demonstrate that tissue-engineered skin with superficial fibroblasts and keratinocytes forms significantly better basement membrane with higher expression of dermo-epidermal adhesive and anchoring proteins, and superior epidermis with enhanced barrier function compared to that with deep fibroblasts and keratinocytes, or with superficial fibroblasts, deep fibroblasts, and keratinocytes. The specific use of superficial fibroblasts in tissue-engineered skin may thus be more beneficial to promote adhesion of newly formed skin and wound healing, and is therefore promising for the treatment of patients with basement membrane disorders and other skin blistering diseases.

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Section: Introductionmentioning

confidence: 99%

Superficial Dermal Fibroblasts Enhance Basement Membrane and Epidermal Barrier Formation in Tissue-Engineered Skin: Implications for Treatment of Skin Basement Membrane Disorders

Varkey

Ding

Tredget

2013

Tissue Engineering Part A

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“…The cases of subepidermal blistering disease associated with more than two antigens have rarely been reported [2,3,4,5]. We describe a case of a subepidermal blistering disease associated with autoantibodies to both type VII collagen and anti-p200 pemphigoid antigen (p200) which was accompanied with exfoliative esophagitis.…”

Section: Introductionmentioning

confidence: 99%

A Case of Epidermolysis Bullosa Acquisita with Autoantibody to Anti-p200 Pemphigoid Antigen and Exfoliative Esophagitis

et al. 2006

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Background: Some cases of a subepidermal blistering disease associated with autoantibodies to more than two antigens have been reported. Observation: A 52-year-old Japanese woman had pruritic blisters on almost the whole body as well as erosive lesions in the oral cavity and esophagus. A histological finding was subepidermal bullae. Direct immunofluorescence (IF) revealed a linear deposition of IgG, IgM and C3 at the epidermal basement membrane zone (BMZ). Indirect IF using human skin split by 1 M NaCl as a substrate showed IgG antibody reactive with the dermal side. By immunoblot analysis using normal human dermal extract, the 200-kDa and 290-kDa bands were detected. Indirect IF did not show any anti-BMZ antibody activity, when using the skin of the patient with recessive dystrophic epidermolysis bullosa as a substrate. Conclusion: We regarded our case as epidermolysis bullosa acquisita with autoantibody to anti-p200 pemphigoid antigen. This is the second case in the literature associated with autoantibodies to these two antigens.

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“…BSLE patients produce autoantibodies that recognize type VII collagen, a major component of anchoring fibrils, which play an important role in dermo-epidermal adhesion. Chan and colleagues 4 identified further autoantibodies reacting to multiple basement membrane components including bullous pemphigoid antigen 1, laminin-5 and laminin-6 in patients with BSLE. This hyper-immune state seems to be associated with the gene for major histocompatibility complex HLA-DR2.…”

Section: Discussionmentioning

confidence: 99%

Bullous Systemic Lupus Erythematosus

et al. 2018

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Abstract Abstract AbstractBullous systemic lupus erythematosus (BSLE) is extremely rare but distinct disease, characterized by vesicobullous skin eruptions in systemic lupus erythematosus (SLE). It can develop either before or after a diagnosis of SLE has been established. BSLE is characterized by a dermatitis herpetiformis-like histology and an autoimmunity to type VII collagen. It must be differentiated from other autoimmune vesicobullous diseases such as epidermolysis bullosa acquisita, dermatitis herpetiformis, linear IgA disease, and bullous pemphigoid. Its important to combine clinical, histological, and immunofluorescence findings to establish a diagnosis of BSLE. We report a case of BSLE to illustrate and emphasize the need for an integrative diagnostic approach.

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Bullous Systemic Lupus Erythematosus With Autoantibodies Recognizing Multiple Skin Basement Membrane Components, Bullous Pemphigoid Antigen 1, Laminin-5, Laminin-6, and Type VII Collagen

Cited by 113 publications

References 35 publications

Superficial Dermal Fibroblasts Enhance Basement Membrane and Epidermal Barrier Formation in Tissue-Engineered Skin: Implications for Treatment of Skin Basement Membrane Disorders

Superficial Dermal Fibroblasts Enhance Basement Membrane and Epidermal Barrier Formation in Tissue-Engineered Skin: Implications for Treatment of Skin Basement Membrane Disorders

A Case of Epidermolysis Bullosa Acquisita with Autoantibody to Anti-p200 Pemphigoid Antigen and Exfoliative Esophagitis

Bullous Systemic Lupus Erythematosus

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